Israel Medical Association Journal

Hypokalemia is a frequently encountered electrolyte disturbance, particularly among hospitalized patients. It often arises from increased potassium excretion (via the kidney or gastrointestinal tract) or intracellular shifts [1]. Hypokalemic periodic paralysis (HPP) is an uncommon cause of hypokalemia, with the most common reported mutation found in the dihydropyridine-sensitive calcium channel in skeletal muscles (CACNA1S gene) [2]. We present a case of a young woman with HPP associated with a novel mutation in the chloride voltage-gated channel 1 (CLCN1) gene.

Inflammatory myopathies include polymyositis, necrotizing autoimmune myositis, dermatomyositis, juvenile inflammatory myopathy, and inclusion body myositis. These diseases are classified based on the different clinical and pathological characteristics unique to each of them [1]. Dermatomyositis is a rare disease with an incidence of 6–10 cases/1,000,000 a year with the highest incidence in the 7th decade of life as reported by a Norwegian cohort in a Caucasian population [2].

Diagnosis of dermatomyositis is based on typical signs and symptoms combined with laboratory results, imaging, and electromyography findings and muscle biopsy. Historically, the diagnosis of dermatomyositis was based on the classification criteria named after Bohan and Peter published in 1975. Many other classification criteria were proposed subsequently, the latter by the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR), which were published in 2020 [3].

The clinical features of dermatomyositis are diverse. Skin manifestations can accompany or precede muscle weakness. Classical skin findings include periorbital heliotrope rash and a rash of the upper chest, back, and shoulders, known as the V sign and shawl sign respectively, as well as the Gottron's papules on the knuckles. Another skin appearance is subcutaneous calcifications that break periodically through the skin causing ulcerations. Dermatomyositis usually manifests as a symmetrical proximal muscle weakness but can present with preserved strength called amyopathic dermatomyositis [1].

Background: The French AmbUlatory Cesarean Section (FAUCS) technique was introduced to the Galilee Medical Center in September 2021. FAUCS was performed electively for interested women who meet the criteria.

Objectives: To evaluate the learning curve of senior surgeons performing FAUCS, the procedure short-term outcomes, and complications.

Methods: This retrospective study included 50 consecutive women who underwent FAUCS from September 2021 until March 2022 at our facility. Preoperative, intraoperative, postoperative, and demographic data were retrieved from patient electronic charts.

Results: The mean duration of surgery was 53.26 ± 11.62 minutes. This time decreased as the surgical team's experience increased: from a mean 58.26 ± 12.25 minutes for the first 15 procedures to a mean 51.17 ± 9.73 minutes for subsequent procedures. The mean visual analogue scale score for 24 hours was 1.08 ± 0.84 (on a 10-point scale). The rate of neonatal cord pH < 7.2 was 6%, and there were 11.3% cases of vacuum assisted fetal extraction. In total, 44% of the women were able to mobilize and urinate spontaneously by 4–6 hours. Complications included bladder injury (n=1), endometritis (n=1), and incisional hematoma (n=1). Overall, the maternal satisfaction rate was high; 94% of the women would recommend FAUCS to others.

Conclusions: FAUCS is a feasible procedure with a high satisfaction rate. Following the first 15 procedures performed by one surgical team, the operative time decreased considerably. Further randomized controlled studies are needed to compare this procedure to regular cesarean section and evaluate neonatal outcomes.

Background: Fibromyalgia syndrome (FMS) is characterized by widespread musculoskeletal pain and tenderness with associated neuropsychological symptoms such as fatigue, unrefreshing sleep, cognitive dysfunction, anxiety, and depression. Osteoporosis is defined as a reduction of bone density. Previous studies to determine an association of FMS with osteoporosis showed mixed results, partially due to small sample sizes and lack of statistical power.

Objectives: To evaluate the association of FMS with osteoporosis.

Methods: We conducted a case-control study utilizing the database from Israel’s largest health maintenance organization. FMS patients were compared to age- and sex-matched controls. Data were analyzed using chi-square and t-tests. Multivariable logistic regression models assessed the association between osteoporosis and FMS. Spearman’s rho test was used for correlation.

Results: We utilized data from 14,296 FMS patients and 71,324 age- and sex-matched controls. Spearman's rho test showed a significant correlation between FMS and osteoporosis (correlation coefficient 0.55, P < 0.001). A logistic regression for osteoporosis showed an odds ratio [OR] of 1.94 (95% confidence interval [95%CI] 1.83–2.06, P < 0.001) for FMS compared to controls and found higher body mass index to be slight protective (OR 0.926, 95%CI 0.92–0.93, P < 0.001).

Conclusions: There is a significant correlation between FMS and osteoporosis. Early detection of predisposing factors for osteoporosis in FMS patients and implementation of suitable treatments and prevention measures (such as dietary supplements, resistance or weight bearing exercise, and bone-mineral enhancing pharmacological therapy) may reduce both occurrence rate and severity of osteoporosis and its complications, such as fractures.

Background: The CHA₂DS₂-VASc score has been shown to predict systemic thromboembolism and mortality in certain groups in sinus rhythm (SR), similar to its predictive value with atrial fibrillation (AF).

Objectives: To compare factors of inflammation, thrombosis, platelet reactivity, and turnover in patients with high versus low CHA₂DS₂-VASc score in SR.

Methods: We enrolled consecutive patients in SR and no history of AF. Blood samples were collected for neutrophil-to-lymphocyte ratio (NLR), C-reactive protein (CRP), immature platelet fraction (IPF%) and count (IPC), CD40 ligand, soluble P-selectin (sP-selectin) and E-selectin. IPF was measured by autoanalyzer and the other factors by ELISA.

Results: The study comprised 108 patients (age 58 ± 18 years, 63 women (58%), 28 (26%) with diabetes), In addition, 52 had high CHA₂DS₂-VASc score (³ 2 for male and ³ 3 for female) and 56 had low score. Patients with low scores were younger, with fewer co-morbidities, and smaller left atrial size. sP-selectin was higher in the high CHA₂DS₂-VASc group (45, interquartile ratio [IQR] 36–49) vs. 37 (IQR 28–46) ng/ml, P = 0.041]. Inflammatory markers were also elevated, CRP 3.1 mg/L (IQR 1.7–9.3) vs. 1.6 (IQR 0.78–5.4), P < 0.001; NLR 2.7 (IQR 2.1–3.8) vs. 2.1 (IQR 1.6–2.5), P = 0.001, respectively. There was no difference in E-selectin, CD40 ligand, IPC, or IPF% between the groups.

Conclusions: Patients in SR with high CHA₂DS₂-VASc score have higher inflammatory markers and sP-selectin. These findings may explain the higher rate of adverse cardiovascular events associated with elevated CHA₂DS₂-VASc score.

Background: The purpose of mastectomy for the transgender patient is to produce a masculine appearance of the chest. A number of algorithms have been proposed for selecting the surgical technique. A holistic and surgical approach to transgender men includes our experience-based classification system for selecting the correct surgical technique.

Objectives: To present and discuss the Transgender Standard of Care and our personal experience.

Methods: Data were collected from the files of female-to-male transgender persons who underwent surgery during 2003–2019. Pictures of the patients were also analyzed.

Results: Until May 2021, 342 mastectomies were performed by the senior author on 171 patients. The 220 mastectomies performed on 110 patients until November 2019 were included in our cohort. Patient age was 13.5 to 50 years (mean 22.5 ± 6.1). The excision averaged 443 grams per breast (range 85–2550). A periareolar approach was performed in 14 (12.7%), omega-shaped resection (nipple-areola complex on scar) in 2 (1.8%), spindle-shaped mastectomy with a dermal nipple-areola complex flap approach in 38 (34.5%), and a complete mastectomy with a free nipple-areola complex graft in 56 (50.9%). Complications included two hypertrophic scars, six hematomas requiring revision surgery, three wound dehiscences, and three cases of partial nipple necrosis.

Conclusions: A holistic approach to transgender healthcare is presented based on the World Professional Association for Transgender Health standard of care. Analysis of the data led to Wolf's classification for female-to-male transgender mastectomy based on skin excess and the distance between the original and the planned position of the nipple-areola complex

Background: In rheumatoid arthritis (RA), females usually have a worse prognosis. To date, the influence of physician gender in the evaluation of RA activity is still largely unknown.

Objectives: To investigate the discrepancy in RA disease activity assessment between male and female physicians and to compare patient and evaluator perception of disease activity and global health (GH) status.

Methods: One female and one male rheumatologist evaluated 154 RA patients recording tender and swollen joint count, GH, evaluator global assessment (EGA), and patient global assessment (PGA) disease activity. A third rheumatologist calculated DAS28, CDAI, and SDAI. Difference was evaluated by Wilcoxon test. Physician–patient agreement was assessed by intraclass correlation coefficient.

Results: GH, PGA, and DAS28 were higher when recorded by the female examiner. Male EGA was higher than female. Among male patients, PGA was higher when collected by the female examiner. The probability of being judged as having an active disease did not rely on physician gender. The agreement with the physician’s evaluation of disease activity was high. PGA values were higher than EGA in both examiners. The physician–patient agreement was moderate for the male examiner and good for the female. The female physician had a higher agreement with both genders.

Conclusions: Subjective measure of disease activity differs between female and male rheumatologists, contributing to a different evaluation of disease activity. Patients have a higher perception of disease activity compared to physicians. The stronger agreement between female physicians and patients may be related to a more emphatic setting established by the female physician

Background: Oral anticoagulants (OAC) reduce the risk for stroke and death from all causes in patients with non-valvular atrial fibrillation (NVAF)

Objective: To explore adherence rates of OAC among patients with NVAF in long-term use in a real-world setting and to examine patient characteristics associated with good adherence.

Methods: We conducted a population-based cohort study with members of Clalit Health Services, Israel. All patients aged ≥ 30 years with a diagnosis of NVAF before 2016 who were treated with OAC were included. We included patients who filled at least one prescription per year in the three consecutive years 2016–2018. We analyzed all prescriptions that were filled for the medications from 1 January 2017 to 31 December 2017. We considered purchasing of at least nine monthly prescriptions during 2017 as good medication adherence.

Results: We identified 26,029 patients with NVAF who were treated with OAC; 10,284 (39.5%) were treated with apixaban, 6321 (24.3%) with warfarin, 6290 (24.1%) with rivaroxaban, and 3134 (12.0%) with dabigatran. Rates of good medication adherence were 88.9% for rivaroxaban, 84.9% for apixaban, 83.6% for dabigatran, and 55.8% for warfarin (P < 0.0001). Advanced age was associated with higher adherence rates (P < 0.001). Socioeconomic status was not associated with medication adherence. Good adherence with OAC was associated with lower low density lipoprotein (LDL) cholesterol and glucose levels.

Conclusions: Adherence rates to OAC in chronic use among patients with chronic NAVF are high. Investing in OAC adherence may have a wider health impact than expected.

Background: Psoriatic arthritis (PsA) is an inflammatory rheumatic disease characterized by different phenotypes in terms of joint involvement. The so-called oligoarticular pattern involves fewer than five active joints at a different time points. The evaluation of disease activity in this subset of patients is an unmet need due to the lack of specific indices able to capture modifications over time.

Objectives: To evaluate the ability of musculoskeletal ultrasound to monitor the response to apremilast treatment in oligoarticular PsA patients.

Methods: We evaluated 24 oligoarticular patients (19 women, 5 men; median age 56 years, interquartile range (IQR) 19; median disease duration 5 years, IQR 5.75). All patients were assessed at baseline (T0), and after 6 (T1), 12 (T2), and 24 (T3) weeks. Clinical assessment included evaluation of 66 swollen joints and patient global health assessment. All the patients underwent ultrasound assessment of the clinically involved joints. Synovial effusion/hypertrophy and power Doppler were scored with a semi-quantitative scale (0–3). The total inflammatory score was the sum of the scores.

Results: We found a reduction in the ultrasound inflammatory score at all time points, with a significant improvement at 6 and 12 weeks of treatment compared with baseline: T0 median 8.5 (IQR 5.0); T1 3.5 (3.0); T2 2.0 (3.5); P  = 0.01. We observed a significant reduction of patient global health assessment after 24 weeks (T0 median 50 (32.5); T3 40 (57.5); P = 0.01).

Conclusions: Musculoskeletal ultrasound could be useful in the assessment of treatment response in PsA patients with oligoarticular subset

Background: The potential excess flow of patients into emergency departments and community clinics for testing and examination during a pandemic poses a major issue. These additional patients may lead to the risk of viral transmission to other patients and medical teams. To contain the spread of coronavirus disease-2019 (COVID-19), the Israeli Ministry of Health initiated a plan spearheaded by Magen David Adom (MDA), Israel’s national emergency medical services (EMS) organization.

Objectives: To describe outbreak containment actions initiated by MDA, including a COVID-19 tele-triage center and home testing by paramedics.

Methods: Retrospective analysis was conducted of de-identified data from the call management and command and control systems during the first period of the COVID-19 outbreak in Israel (23 February 2020–15 March 2020).

Results: During the study period, the total number of calls to the dispatch centers was 477,321 with a daily average of 21,696, compared to 6000–6500 during routine times. The total number of COVID-19 related calls was 334,230 (daily average 15,194). There were 28,454 calls (8.51% of all COVID-19 related calls, average 1293/day) transferred to the COVID-19 call center. Of the COVID-19 call center inquiries, 8390 resulted in the dispatch of a dedicated vehicle, including a paramedic wearing personal protective equipment, to collect samples for testing (daily average 381).

Conclusions: Maximizing EMS during a pandemic using phone triage, in addition to dispatching paramedics to perform home testing, may significantly distance infected patients from the public and health care system. These steps can further minimize the spread of disease.

Background: Cyclophosphamide treatment has been associated with ovarian function impairment. Co-treatment with gonadotropin-releasing hormone-analogue (GnRH-a) seems to be able to prevent this complication. However, even though data are available on neoplastic patients, limited data have been published on systemic lupus erythematosus (SLE) women cohorts

Objectives: To evaluate GnRH-a efficacy on ovarian function preservation in SLE women receiving cyclophosphamide treatment

Methods: The authors performed a retrospective study including SLE women requiring cyclophosphamide treatment and compared those treated with and without GnRH-a (case and controls, respectively). All patients were evaluated before cyclophosphamide treatment and every 3 months in the following years. Ovarian function was evaluated using hormonal profiles

Results: The study comprised 33 SLE cyclophosphamide-treated women: 18 co-treated with triptorelin and 15 controls. The mean follow-up was 8.1 ± 5.1 years (range 4–11). Premature ovarian failure (POF) prevalence was significantly lower in SLE women treated by cyclophosphamide plus triptorelin compared to controls (11.1% vs. 33.3%, P = 0.0002). The occurrence of POF was significantly associated with higher age at the time of cyclophosphamide treatment (P = 0.008). Only patients in the GnRH-a treated group had successful pregnancies

Conclusions: The study provides information about the efficacy of co-treatment with GnRH-a in SLE women receiving cyclophosphamide, as demonstrated by the lower POF incidence compared to untreated subjects, based on long-term follow-up. These results reinforce the use of GnRH-a for fertility preservation in premenopausal SLE patients treated by cyclophosphamide

Background: Heart failure centers with specialized nurse-supervised management programs have been proposed to improve prognosis. The Heart Failure Center in Beit Shemesh, Israel, is located within a large primary care facility. The specialist team supervised the managememt of patients both within the frame of the center and while they were hospitalized.

Objectives: To evaluate the health services utilization by heart failure patients treated at a heart failure center and their clinical outcome.

Methods: In this retrospective study, we compared the clinical outcome of patients treated at a heart failure center to patients who received the standard care in 2013–2014. The clinical outcome included primary care visits, emergency room visits, hospitalizations, and death.

Results: The study comprised 430 heart failure patients; 82 were treated at the heart failure center and 348 under standard care. At baseline, no significant differences were seen in clinical parameters between the groups. Healthcare utilization was higher among the study group. No significant changes in healthcare utilization were found. During follow-up, patients treated in a heart failure center were more likely to get recommended heart failure medications. Mortality was significantly lower in patients treated in the heart failure center compared with those receiving standard care 3.6% vs. 24%, respectively (P = 0.001), hazard ratio 0.19, 95% confidence interval 0.06–0.62, P = 0.005.

Conclusions: Joint management of heart failure by primary clinics and a specialized community heart failure center reduced mortality. There was no decrease in healthcare utilizations among heart failure center patients, despite the reduction in mortality.